Diagnosis and Management of Global Development Delay: Consensus Guidelines of Growth, Development and Behavioral Pediatrics Chapter, Neurology Chapter and Neurodevelopment Pediatrics Chapter of the Indian Academy of Pediatrics

Justification: Global developmental delay (GDD) is a relatively common neurodevelopmental disorder; however, paucity of published literature and absence of uniform guidelines increases the complexity of clinical management of this condition. Hence, there is a need of practical guidelines for the pediatrician on the diagnosis and management of GDD, summarizing the available evidence, and filling in the gaps in existing knowledge and practices. Process: Seven subcommittees of subject experts comprising of writing and expert group from among members of Indian Academy of Pediatrics (IAP) and its chapters of Neurology, Neurodevelopment Pediatrics and Growth Development and Behavioral Pediatrics were constituted, who reviewed literature, developed key questions and prepared the first draft on guidelines after multiple rounds of discussion. The guidelines were then discussed by the whole group in an online meeting. The points of contention were discussed and a general consensus was arrived at, after which final guidelines were drafted by the writing group and approved by all contributors. The guidelines were then approved by the Executive Board of IAP. Guidelines: GDD is defined as significant delay (at least 2 standard deviations below the mean with standardized developmental tests) in at least two developmental domains in children under 5 years of age; however, children whose delay can be explained primarily by motor issues or severe uncorrected visual/ hearing impairment are excluded. Severity of GDD can be classified as mild, moderate, severe and profound on adaptive functioning. For all children, in addition to routine surveillance, developmental screening using standardized tools should be done at 9-12 months,18-24 months, and at school entry; whereas, for high risk infants, it should be done 6-monthly till 24 months and yearly till 5 years of age; in addition to once at school entry. All children, especially those diagnosed with GDD, should be screened for ASD at 18-24 months, and if screen negative, again at 3 years of age. It is recommended that investigations should always follow a careful history and examination to plan targeted testing and, vision and hearing screening should be done in all cases prior to standardized tests of development. Neuroimaging, preferably magnetic resonance imaging of the brain, should be obtained when specific clinical indicators are present. Biochemical and metabolic investigations should be targeted towards identifying treatable conditions and genetic tests are recommended in presence of clinical suspicion of a genetic syndrome and/or in the absence of a clear etiology. Multidisciplinary intervention should be initiated soon after the delay is recognized even before a formal diagnosis is made, and early intervention for high risk infants should start in the nursery with developmentally supportive care. Detailed structured counselling of family regarding the diagnosis, etiology, comorbidities, investigations, management, prognosis and follow-up is recommended. Regular targeted follow-up should be done, preferably in consultation with a team of experts led by a developmental pediatrician/ pediatric neurologist.

Drug utilization study in schizophrenia patients in outpatient department of psychiatry: an observational prospective study

Background: A mental disorder or psychiatry disorder is a behavioural or mental pattern that causes significant distress or impairment of personal functioning. Schizophrenia is one of the most common psychiatric disorders. Aim of the study is to study drug utilization pattern in schizophrenia patients in outpatient department of psychiatry.Methods: A prospective and observational study was conducted in psychiatry outpatient department between May 2018 to October 2018 at Government Medical College and Hospital Aurangabad. Approval from ethics committee was taken. Data collected was of age, sex, final diagnosis, drugs prescribed to patients from case report form. The data obtained was then analyzed in Microsoft excel.Results: Total prescriptions of 129 patients were analyzed. Out of 129 patients 99 (76.74%) were male, while 30 (23.26%) were female. The mean age of the patients admitted in Psychiatry OPD was 36.71±11.76 years. Number of drugs prescribed per patient being 3.40±1.12 (mean±SD). The most common drugs used were olanzapine (66.6%), trihexyphenidyl (51.9%), lorazepam (44.9%), risperidone (39.5%), trifluoperazine (27.9%).Conclusions: Our study shows that atypical antipsychotics were the most common drugs prescribed in patients. Olanzapine was the most common medication used followed by risperidone. Our prospective study is carried out to get a more comprehensive data so that we can improve the drug utilization in our hospital.

Drug utilization study in medicine intensive care unit in tertiary care hospital

Background: The medical intensive care unit (MICU) is a setting were patients who are critically ill are admitted and thus usually receive a large number of drugs of different pharmacological classes due to life threatening illnesses which may be fatal. The various drugs used in MICU and there clinical outcome was investigated in this study.Methods: Patients admitted between January 2017 to June 2018 in Medicine ICU of GMC Aurangabad were included in this study.Results: Total of 351 prescriptions was analyzed from the medicine intensive care unit. Out of 351 patients 243 (69.23%) were male, while 108 (30.77%) were female. The mean age of the patients admitted in MICU was 42.78�.14 years. The most common type of patients admitted in MICU have the diagnosis of organophosphorous poisoning (25.36%), followed by cerebrovascular accident (15.95%), pneumonitis (10.26%), snake bite (7.12%), chronic kidney disease (5.98%), diabetic ketoacidosis (5.70%) and seizure disorder (3.42%). The most common drugs used were ranitidine (99.71%) and ondansetron (99.43%). Among antibiotics cephalosporins were most commonly used, ceftriaxone (39.03%), cefotaxim (40.46%), cefoperazone and sulbactum (24.22%). Other antibiotics most commonly used were metronidazole (61.25%), amoxicillin and potassium clavulanate (26.78%), piperacillin and tazobactum (11.68%), meropenem (11.40%).Conclusions: In conclusion, we found that in MICU utilization rate of gastroprotective (ranitidine), antiemetic (ondansetron) and antibiotics (cephalosporins) was high. The present study provides valuable insight about the overall pattern of drugs used in medicine intensive care unit.

Salmonella Paratyphi B bacteremia in Systemic Lupus Erythematosus with an unusual presentation- a case report.

Background- Systemic Lupus Erythematosus (SLE) is an inflammatory and multisystem autoimmune disorder. Patients of SLE are at increased risk of infections owing to underlying immunological derangements and to the use of therapeutic regimens like immunosuppressive agents. Among the bacterial infections presenting as bacteremia in these patients, non typhoidal and typhoidal salmonellosis are commonly encountered. We report a rare case of Salmonella Paratyphi B bacteremia in a patient with juvenile onset SLE on treatment with corticosteroids.

Drugs in pregnancy and lactation.

Drug use during pregnancy and lactation is an almost inevitable event. Some of the drugs may have adverse effects on the baby on exposure. It is therefore necessary for the physician to be aware of the changes in pharmacokinetics and pharmacodynamics during pregnancy. Knowledge about the known teratogens and drugs safe to be used during pregnancy is essential on the part of the prescribing physician. There are many factors affecting drug passage into the milk and thus affecting the child in some cases. There are also some drugs affecting lactation. All this data needs to be carefully studied by the physician.

Septicemia due to ESBL producing klebsiella pneumoniae in multi transfused thalassemic patient with splenectomy.

The spleen plays a major role in the opsonisation of capsulated bacteria. A thalassemic patient is treated with prophylactic antibiotics, vaccines, chelating agents, blood transfusions and many a time, with splenectomy. The surgery predisposes patients to infections with capsulated organisms.Vaccines can prevent occurrence of streptococcal and haemophilus infections to some extent. However Gram negative septicaemia due to capsulated bacteria can be overwhelming in these patients and when caused by multidrug resistant strains can warrant the need for administration of expensive antibiotics to save the life of the patient.

Managing Specific Learning Disability in Schools in India.

Specific learning disability (dyslexia, dysgraphia, and dyscalculia) afflicts 5-15% of school-going children. Over the last decade; awareness about this invisible handicap has grown in India. However, much needs to be done to ensure that each afflicted child gets an opportunity to achieve his or her full academic potential in regular mainstream schools. In order to achieve this ideal scenario, all ‘regular’ classroom teachers should be sensitized to suspect, and trained to screen for this disability when the child is in primary school. School managements should become proactive to set up resource rooms and employ special educators to ensure that these children receive regular and affordable remedial education; and be diligent in ensuring that these children get the mandatory provisions both during school and board examinations. Once specific learning disability is recognized as a disability by the Government of India, these children with the backing of the Right to Education Act, would be able to benefit significantly.

Recollections of learning-disabled adolescents of their schooling experiences : A qualitative study.

Background: Little is known about the actual impact of the schooling experience on adolescents with specific learning disability (SpLD). Aims: To analyze the recollections of adolescents with SpLD who were undergoing education in regular mainstream schools. Settings And Design: Prospective interview-based study conducted in our clinic. Materials And Methods: Adolescents' responses to a semi-structured interview were audio-taped, transcribed verbatim and interpreted by content analysis. The number of participants was determined by 'saturation sampling,' resulting in a total of 30 adolescents. Results: Twelve (40%) adolescents had 'overall' neutral recollections, 9 (30%) had 'overall' positive recollections and 9 (30%) had 'overall' negative recollections about having SpLD during their schooling. Fourteen (46.7%) adolescents stated 'getting provisions' as good features, whereas 7 (23.3%) stated 'feeling different from classmates' and 6 (20%) stated 'being teased by classmates about their disability' as bad features of having SpLD. Nineteen (63.3%) adolescents remembered classroom teachers being supportive, while 7 (23.3%) remembered being insulted by them. Of the 21 adolescents who had undergone remedial education, only 10 (47.6%) acknowledged that it had benefited them. Twenty-one (70%) adolescents stated that availing provisions helped in getting better marks in examinations. Ten (33.3%) adolescents had negative recollections about their parents' behavior in relation to their disability. Twenty (66.7%) adolescents wanted changes in their school to help students with SpLD. Conclusion: Improving the knowledge of classroom teachers, classmates and family members about SpLD and about the rationale of provisions will help reduce the unpleasant experiences students with SpLD undergo during their schooling years.

Bone metabolism alteration on antiepileptic drug therapy.

OBJECTIVE: To investigate whether serum total alkaline phosphatase (ALP), bone-specific ALP (bone ALP), calcium, phosphorus, 25-hydroxyvitamin D (25-OHvit D) concentrations are altered early in the course of treatment with carbamazepine or valproic acid monotherapy in ambulatory children with adequate sun exposure; and to determine the effectiveness of simultaneous supplementation with calcium and 25-OHvit D at recommended dietary allowance doses on these biochemical parameters. METHODS: For each drug, children were divided into two groups (Group A: without supplementation; and Group B: with supplementation) and serum biochemical parameters estimated at 0, 30, 60, and 90 days of starting treatment. Statistical analysis: Serial changes in serum biochemical parameters (mean +/- SD) were compared within each of the four groups using student's paired t test. Also for each drug, serum biochemical parameters were compared between Groups A and B at 0, 30, 60, and 90 days of starting treatment using student's unpaired t test. RESULTS: For both drugs, in Group A, serum total ALP levels were significantly increased above the normal range (P<0.0001) by 90 days of starting treatment; however, serum bone ALP level was significantly increased (P=0.002) only in children on valproic acid. For both drugs when serum biochemical parameters were compared between Groups A and B, supplementation resulted in a significant decrease in serum total ALP (P<0.0001) and bone ALP levels (P<0.001), and a significant increase in serum calcium (P<0.0001) and 25-OHvit D levels (P<0.0001) by 90 days of starting treatment. CONCLUSION: Serum biochemical changes which indicate predisposition to development of rickets or osteomalacia appear within 90 days of starting carbamazepine or valproic acid monotherapy. However simultaneous supplementation with oral calcium and 25-OHvit D is effective in preventing the development of these adverse biochemical changes.

Impact of an educational program on parental knowledge of cerebral palsy.

OBJECTIVES: To investigate parental knowledge of cerebral palsy, and to evaluate the impact of an educational intervention on it. METHODS: From May 2003 to April 2004, 26 parents of newly diagnosed children with cerebral palsy were interviewed. After the interview, each parent was administered a structured educational program and re-interviewed after three months. The pre and post intervention responses were compared using Chi-square test. RESULTS: After the intervention, there was a significant improvement in parental knowledge: (i) of the cause of the disorder (5/26 vs 20/26, P = 0.0001), (ii) that it is non-progressive (16/26 vs 24/26, P = 0.021), (iii) that it is not curable (10/26 vs 23/26, P = 0.0005), (iv) that it is treatable (12/26 vs 24/26, P=0.0009), (v) of the frequency and duration of therapy necessary to improve functional abilities (7/26 vs 17/26, P = 0.005), and, (vi) of the importance of following up regularly with a pediatrician (17/26 vs 26/26, P = 0.003). However, there was no significant improvement in parental knowledge: (i) of the meaning of the term 'cerebral palsy' (0/26 vs 5/26, P = 0.060), (ii) that 'early intervention therapy' given by a team of therapists is its recommended therapy (18/26 v 23/26, P = 0.174), (iii) of the meaning of the term 'early intervention therapy' (12/26 vs 17/26, P = 0.163), and (iv) that it is preventable with good medical care (8/26 vs 10/26, P = 0.560). CONCLUSION: Parental knowledge of cerebral palsy is inadequate. A single-session educational program can significantly improve parental knowledge about many 'core basic issues' regarding cerebral palsy.

Clinical and psychoeducational profile of children with borderline intellectual functioning.

OBJECTIVE: To document the clinical profile and academic history of children with borderline intellectual functioning ("slow learners"); and to assess parental knowledge and attitudes regarding this condition. METHODS: From November 2004 to April 2005, 55 children (35 boys, 20 girls) were diagnosed as slow learners based on current level of academic functioning and global IQ scores (71-84) done by the WISC test. Detailed clinical and academic history; and physical and neurological examination findings were noted. The parents were counseled about the diagnosis and the option of special education. RESULTS: The mean age of slow learners was 11.9 years (+/-SD 2.3, range 8-17). Eighteen (32.7%) children had a significant perinatal history, 15 (27.3%) had delayed walking, 17 (30.9%) had delayed talking, 17 (30.9%) had microcephaly, 34 (61.8%) had presence of soft neurologic signs, and 10 (18.2%) were on complementary and alternative medication therapy. There were no differentiating features between the two gender groups. Their chief academic problems were difficulty in writing (92.7%), overall poor performance in all subjects (89.1%), and difficulty in mathematics (76.4%). Forty-six (83.6%) children had failed in examinations, 34 (61.8%) had experienced grade retention, and 32 (58.2%) had behavior problems. Most parents (83.3%) were reluctant to consider the option of special education. CONCLUSION: Slow learners struggle to cope up with the academic demands of the regular classroom. They need to be identified at an early age and their parents counseled to understand their academic abilities.

Clinical and psychoeducational profile of children with specific learning disability and co-occurring attention-deficit hyperactivity disorder.

BACKGROUND: Almost 10% of school-going children have specific learning disability (SpLD) in the form of dyslexia, dysgraphia and/or dyscalculia. Attention-deficit hyperactivity disorder (ADHD) occurs as a comorbidity in about 20% of these children. AIMS: To document the clinical profile and academic history of children with SpLD and co-occurring ADHD. SETTINGS AND DESIGN: Prospective observational study conducted in our clinic. MATERIALS AND METHODS: From August to November 2004, 50 consecutively diagnosed children (34 boys, 16 girls) were included in the study. SpLD was diagnosed on the basis of psychoeducational testing. Diagnosis of ADHD was made by DSM-IV-revised criteria. Detailed clinical and academic history and physical and neurological examination findings were noted. STATISTICAL ANALYSIS: Chi-square test or unpaired student's t-test was applied wherever applicable. RESULTS: The mean age of children was 11.4 years (+/-SD 2.5, range 7-17.1). Fifteen (30%) children had a significant perinatal history, 12 (24%) had delayed walking, 11 (22%) had delayed talking, 5 (10%) had microcephaly, 27 (54%) displayed soft neurological signs and 10 (20%) had primary nocturnal enuresis. There were no differentiating features between the two gender groups. Their academic problems were difficulties in writing (96%), inattentiveness (96%), difficulties in mathematics (74%), hyperactivity (68%) and difficulties in reading (60%). All children had poor school performance, 15 (30%) had already experienced class retention and 20 (40%) had developed aggressive or withdrawn behavior. CONCLUSION: Children with SpLD and co-occurring ADHD need to be identified at an early age to prevent poor school performance and behavioral problems.

Impact of an education program on parental knowledge of specific learning disability.

BACKGROUND: A supportive home environment is one of the factors that can favorably determine the outcome of specific learning disability (SpLD) in a school-going child. However, there is no reliable information available on parental knowledge about SpLD. AIMS: To investigate parental knowledge of SpLD and to evaluate the impact of an educational intervention on it. SETTINGS AND DESIGN: Prospective questionnaire-based study conducted in our clinic. MATERIALS AND METHODS: From April to November 2002, 50 parents who were conversant in English and willing to follow up were interviewed. After the interview, each parent was administered a structured educational program and re-interviewed after 3 months. STATISTICAL ANALYSIS: The pre- and post-intervention responses were compared using Chi-square test. RESULTS: After the intervention, there was significant improvement in parental knowledge of (i) the meaning of the term 'SpLD' (32/50 vs. 50/50, P CONCLUSION: Parental knowledge of their child's SpLD is inadequate and this can be significantly improved by a single-session educational program.

Fanconi-Bickel syndrome.

We present here the first case of Fanconi-Bickel syndrome, a rare type of glycogen storage disease, from India. A 17-month-old female child presented with severe growth retardation and abdominal distention. Clinical examination revealed a "doll-like" face, massive hepatomegaly, and rickets. Laboratory investigations confirmed severe hypophosphatemic rickets and proximal renal tubular dysfunction. Liver biopsy showed glycogen accumulation in the hepatocytes.

Educational provisions and learning disability.

OBJECTIVE: To assess the impact of the provisions of the Maharashtra government on the academic performance of children with specific learning disability (SpLD) at the Secondary School Certificate (SSC) board examination. METHODS: The academic performance of 60 children (45 boys, 15 girls) at the SSC board examination with benefit of chosen provisions was compared with their performance at their last annual school examination before diagnosis of SpLD. RESULTS: There was a significant improvement in their mean percentage (%) total marks scored at the SSC board examination as compared with the mean % total marks scored by them at their last annual school examination before the diagnosis (63.48 +/- 7.86 vs. 40.95 +/- 7.23) [mean +/- SD, mean % difference = 22.53, 95% CI = 19.8 - 25.26, P. CONCLUSION: Children with SpLD who availed the benefit of provisions showed a significant improvement in their academic performance at the SSC board examination.

Cognition in specific learning disability.

OBJECTIVE: To compare the cognition abilities of children with specific learning disability (SpLD) viz. dyslexia, dysgraphia and dyscalculia with those of non-impaired children. METHODS: The study group consisted of 95 newly diagnosed SpLD children (aged 9-14 years) and the control group consisted of 125 non-impaired children (aged 9-14 years). An academic achievement of two years below the actual grade placement on educational assessment with a Curriculum-Based test was considered diagnostic of SpLD. A battery of 13 cognition function tests based on Guilford's Structure of Intellect Model was administered individually on each child in four areas of information viz. figural, symbolic, semantic and behavioral. Mean scores +/- SD obtained in these four areas were calculated in both groups and compared using Independent Samples t-test. A P value < 0.05 was considered significant. RESULTS: Children with SpLD had significantly lower scores (mean +/- SD) in all four areas of information: maximally in the symbolic area (18.66 +/- 4.83 vs. 28.30 +/- 4.29, mean difference 9.64, P < 0.0001, df = 218, 95% CI 8.43-10.86), followed by semantic (18.72 +/- 5.07 vs 27.36 +/- 4.17, mean difference 8.64, P < 0.0001, df = 218, 95% CI 7.40-9.87), figural (17.10 +/- 5.24 vs 25.14 +/- 3.36, mean difference 8.04, P < 0.0001, df = 218, 95% CI 6.89-9.19), and behavioral (5.68 +/- 2.10 vs 7.54 +/- 1.46, mean difference 1.86, P < 0.0001, df = 218, 95% CI 1.39-2.33) areas. CONCLUSION: Cognition abilities are significantly impaired in children with SpLD.

Poor school performance.

Education is one of the most important aspects of human resource development. Poor school performance not only results in the child having a low self-esteem, but also causes significant stress to the parents. There are many reasons for children to under perform at school, such as, medical problems, below average intelligence, specific learning disability, attention deficit hyperactivity disorder, emotional problems, poor socio-cultural home environment, psychiatric disorders and even environmental causes. The information provided by the parents, classroom teacher and school counselor about the child's academic difficulties guides the pediatrician to form an initial diagnosis. However, a multidisciplinary evaluation by an ophthalmologist, otolaryngologist, counselor, clinical psychologist, special educator, and child psychiatrist is usually necessary before making the final diagnosis. It is important to find the reason(s) for a child's poor school performance and come up with a treatment plan early so that the child can perform up to full potential.

Rett syndrome: a case report and overview.

A 3-year-old girl with Rett syndrome is reported in this case report. The aim of this report is to highlight the clinical features of this rare neurodegenerative disorder that mainly affects young girls. A brief review of literature of this disorder is included.

Tuberculous meningitis and HIV.

OBJECTIVE: To identify factors associated with HIV-infected status in children admitted with tuberculous meningitis (TBM), and to find out whether HIV co-infection affects in-hospital outcome. METHODS: This prospective hospital-based study was conducted from May 2000 to August 2003. All consecutive children, aged 1 month to 12 years of age, admitted with a diagnosis of TBM were enrolled. Relationship between 35 features viz., two demographic factors, nine clinical features, 13 neurological features, five laboratory (including cerebrospinal fluid) parameters, six radiological (including computed tomography scan brain) features, and the two outcomes (disabled survivor or death); with HIV-infected status was assessed. RESULTS: Of a total 123 TBM cases enrolled, eight (6.5%) were HIV-infected. There was no significant difference between the two groups, except that more children in the HIV-infected group had Hb < 8 gm/dl: both on bivariate analysis, (OR, 12.0; 95% CI, 2.6-55.9; P = 0.001) and on multivariate analysis (OR, 12.30; 95% CI, 1.9-79.6; P = 0.008). Outcome was similar in both the groups. CONCLUSION: Only presence of Hb < 8 gm/dl was associated with HIV-infected status. HIV co-infection did not affect the outcome.

Prognostic clinical variables in childhood tuberculous meningitis: an experience from Mumbai, India.

BACKGROUND: In India, tuberculous meningitis (TBM) is still a major cause of neurological disabilities and death. AIM: To identify the clinical variables which predict the outcome in childhood TBM. SETTING: Tertiary teaching hospital. DESIGN: Prospective observational study. MATERIALS AND METHODS: Thirty-six clinical variables were analyzed in 123 consecutive children with TBM admitted between May 2000 and August 2003. The outcome was assessed in terms of survival or death. Survival meant that the patient was discharged from hospital having made a complete recovery, or with disability. RESULTS: Twenty-five (20%) children recovered completely, 70 (57%) survived with disability, and 28 (23%) died. Employing univariate analysis nine variables correlated with survival with disability outcome: presence of tonic motor posturing, cranial nerve palsy, focal neurological deficit, hypertonia, moderate to severe hydrocephalus, cerebral infarction on cranial CT, and requiring shunt surgery, and absence of extracranial tuberculosis and no antituberculous-related hepatotoxicity; two variables correlated with fatal outcome: presence of deep coma (Glasgow coma scale score P = 0.012, d.f. = 1, OR 0.12, 95% CI 0.02-0.62) correlated with survival with disability outcome, and presence of deep coma (P = 0.030, d.f. = 1, OR 0.35, 95% CI 0.14-0.90) with fatal outcome. CONCLUSION: In children with TBM, the presence of hypertonia at admission is an independent predictor of neurological sequelae in survivors, and deep coma is an independent predictor of mortality.